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L. Angir, M.A., M.D., Ph.D.

Vice Chair, Rocky Vista University College of Osteopathic Medicine

For patients, however, the starting point is on the right, since they experience primarily the societal consequences. The patient may come with a complaint: "Doctor, I cannot read"; the doctor translates this to a statement about the eyes: "She has lost three lines. Recognizing how doctors and patients approach medical conditions from different points of view is essential for effective communication. Clinicians, who are interested in how each eye functions, will measure visual acuity for each eye separately. Although we have two eyes, those eyes are part of a single visual system that generates a single visual perception. This shift in emphasis was explicitly recognized by the World Health Organization in a 2003 consultation, which acknowledged the fact that health statistics are not only a tool to detect eye disease, but also to describe the burden of vision loss in a population. A comprehensive reading assessment should not only determine the smallest print size read, but also reading speed, reading endurance, reading enjoyment, and reading comprehension. Since any rehabilitation requires teamwork involving different professionals to deal with the various components and since vision loss is the common denominator, the ophthalmologist should coordinate the team. For the general ophthalmologist, this may involve only a general question, such as, "How does your vision loss bother you most The possibility of deterioration of vision is best made known from the beginning but must be accompanied with advice about the availability of skilled professionals and resources. Unfortunately, many practitioners are poorly trained in conveying bad news, a skill that should be taught and practiced in medical school. All ophthalmologists should master this skill, which includes informing the patient about options and knowing the appropriate referral sources. Some ophthalmology practices may employ professionals who can provide basic services in-house. For more complex cases, referral to specialized vision rehabilitation services is appropriate. Examination 1025 the standard ophthalmic examination, including identification of any conditions amenable to specific treatment, needs to be adapted as discussed in Chapter 24. Observation of visual performance is important in young children, where regular testing may not be possible. Reports from parents and teachers are often as informative as direct observation in the office. Even for adults, observation of the performance of daily living tasks can be helpful. Questionnaires can assess the subjective difficulty of tasks, including those that cannot be assessed in the office. A disadvantage is that the responses are subjective, with some patients exaggerating their difficulties and others understating them. Assessment of mobility, including identification of peripheral visual field loss, is very important since impaired mobility should trigger referral for assessment by an orientation and mobility (O+M) instructor. Patients also need to be made aware of the importance of appropriate signaling of their visual impairment. Comprehensive Rehabilitation Plans A comprehensive vision rehabilitation plan requires attention to more than just how the eyes function. It is useful to use this as a checklist, although not all parts will be needed in every case. Common examples include talking books and voice-output devices (see Chapter 24), Braille, and long canes. Vision enhancement and vision substitution are not mutually exclusive but complementary. A patient may use a magnifier to read price tags and talking books for recreational reading. A patient with retinitis pigmentosa, who has normal mobility in the daytime, may need a cane at night. Family members, caregivers, and office personnel should be familiar with sightedguide techniques to effectively assist visually impaired patients with minimal embarrassment. They require training of the dog as well as of the patient, who needs to be physically active and able to manage the dog.

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Certain clues, when present, help to differentiate the two entities: acute encephalopathy appears with or shortly after a banal viral infection, usually occurs in children under 5 years of age, may be associated with hypoglycemia and liver function abnormalities, and usually produces only a modest degree of fever. The same reaction can also be triggered by vaccination and rarely by bacterial or parasitic infection. In the first, the invading organism or vaccine is molecularly similar to a brain protein (molecular mimicry), but sufficiently different for the immune system to recognize it as nonself and mount an immune attack against the brain or spinal cord. Because the brain is a relatively immune protected site, the immune system may not have been exposed to the brain protein before and it mounts an immune attack. The disorder largely affects children, but adults and even the elderly are sometimes affected. In parainfectious disseminated encephalomyelitis, the brain and spinal cord contain multiple perivascular zones of demyelination in which axis cylinders may be either spared or destroyed. Clinically, the illness occasionally arises spontaneously, but usually it follows by several days a known or presumed viral infection, frequently an exanthem. In most cases, there is early evidence of behavioral impairment, and as the disorder progresses, the patient may lapse into delirium, stupor, or coma. Both focal and generalized convulsions are common, as are focal motor signs such as hemiplegia or paraplegia. Sometimes gray matter is involved as well as white matter, which may explain the tendency for seizures to occur. The diagnosis of acute disseminated encephalomyelitis should be suspected when a patient becomes neurologically ill following a systemic viral infection or vaccination. Evidence of widespread or multifocal nervous system involvement and of mild lymphocytic meningitis supports the diagnosis. Acute hemorrhagic leukoencephalopathy is considered a variant of encephalomyelitis. These latter vary in diameter from microscopic to several centimeters and are accompanied by focal necrosis and edema. The perivascular infiltrations frequently contain many neutrophils, and there is often perivascular fibrinous impregnation. The illness may follow a banal viral infection or may complicate septic shock, but often no such history isobtained. Affected patients rapidly lapse into coma with high fever but little or no nuchal rigidity. Focal cerebral hemorrhages and edema may produce both the clinical and radiographic signs of a supratentorial mass lesion. Multifocal, Diffuse, and Metabolic Brain Diseases Causing Delirium, Stupor, or Coma 273 As a rule, the problem in the differential diagnosis of coma presented by disseminated and hemorrhagic encephalomyelitis is to distinguish it from viral encephalitis and acute toxic encephalopathy. As a general rule, patients with viral encephalitis tend to be more severely ill and have higher fevers for longer periods of time than patients with disseminated encephalomyelitis, with the exception of the hemorrhagic variety. In one series of 90 brain biopsies for evaluation of dementia, only 57% were diagnostic. Only those specific illnesses that may be perplexing causes of stupor or coma are considered here. Cerebral Biopsy for Diagnosis of Encephalitis When faced with a delirious or stuporous patient suspected of suffering acute encephalitis, the physician is often perplexed about how best to proceed. The clinical pictures of the various forms of encephalitis are often so similar that only cerebral biopsy will distinguish them, but the treatment of the various forms differs. Of the acute viral encephalitides, herpes simplex can be effectively treated by antiviral agents, and it is likely that in some immune-suppressed patients other viral infections such as varicella-zoster and cytomegalovirus also respond to antiviral treatment. The biopsy should be taken either from an involved area, or if the illness is diffuse, from Granulomatous Central Nervous System Angiitis In this acute disorder of the nervous system, the pathologic changes in blood vessels may be limited to the brain or involve other systemic organs. When the disease is limited to the brain, it tends to affect small leptomeningeal and intracerebral blood vessels. Even when the disease is extracerebral, it can affect the blood supply of the brain, producing acute neurologic symptomatology including coma. The disorder has been associated with herpes zoster infection, lymphomas, sarcoidosis, amyloid angiopathy, and infections by mycoplasma, rickettsia, viruses, and Borrelia burgdorferi. Conventional cerebral angiography is more sensitive, but still will only identify irregularity of vessels of 1 mm or larger. In addition, the pattern of irregularity does not verify the underlying pathology, but only indicates areas at which biopsy may be fruitful. The specific diagnosis can only be established by cerebral biopsy, but because the lesions are often multifocal but not diffuse, at times even that fails to demonstrate the pathology.

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The depth or width of a column, for example, is only marginally larger in a primate brain than in a rat brain. The hugely enlarged sheet of cortical columns in a human brain provides the massively parallel processing power needed to perform a sonata on the piano, solve a differential equation, or send a rocket to another planet. An important principle of cortical organization is that neurons in different areas of the cerebral cortex specialize in certain types of operations. In a young brain, before school age, it is possible for cortical functions to reorga- nize themselves to an astonishing degree if one area of cortex is damaged. However, the organization of cortical information processing goes through a series of critical stages during development, in which the maturing cortex gives up a degree of plasticity but demonstrates improved efficiency of processing. Hence, the individual with a large right parietal infarct not only loses the ability to appreciate stimuli from the left side of space, but also loses the concept that there is a left side of space. We have witnessed a patient with a large right parietal lobe tumor who ate only the food on the right side of her plate; when done, she would Pathophysiology of Signs and Symptoms of Coma 27 get up and turn around to the right, until the remaining food appeared on her right side, as she was entirely unable to conceive that the plate or space itself had a left side. Such a patient continues to speak meaningless babble and is surprised that others no longer understand his speech because the very concept that language symbols are embedded in speech eludes him. This concept of fractional loss of consciousness is critical because it explains confusional states caused by focal cortical lesions. It is also a common observation by clinicians that, if the cerebral cortex is damaged in multiple locations by a multifocal disorder, it can eventually cease to function as a whole, producing a state of such severe cognitive impairment as to give the appearance of a global loss of consciousness. During a Wada test, a patient receives an injection of a short-acting barbiturate into the carotid artery to anesthetize one hemisphere so that its role in language can be assessed prior to cortical surgery. When the left hemisphere is acutely anesthetized, the patient gives the appearance of confusion and is typically placid but difficult to test due to the absence of language skills. When the patient recovers, he or she typically is amnestic for the event, as much of memory is encoded verbally. Following a right hemisphere injection, the patient also typically appears to be confused and is unable to orient to his or her surroundings, but can answer simple questions and perform simple commands. The experience also may not be remembered clearly, perhaps because of the sudden inability to encode visuospatial memory. However, the patient does not appear to be unconscious when either hemisphere is acutely anesthetized. An important principle of examining patients with impaired consciousness is that the condition is not caused by a lesion whose acute effects are confined to a single hemisphere. A very large space-occupying lesion may simultaneously damage both hemispheres or may compress the diencephalon, causing impairment of consciousness, but an acute infarct of one hemisphere does not. Hence, loss of consciousness is not a typical feature of unilateral carotid disease unless both hemispheres are supplied by a single carotid artery or the patient has had a subsequent seizure. The concept of the cerebral cortex as a massively parallel processor introduces the question of how all of these parallel streams of information are eventually integrated into a single consciousness, a conundrum that has been called the binding problem. Although most people believe that they experience consciousness in this way, there is no a priori reason why such a self-experience cannot be the neurophysiologic outcome of the massively parallel processing. However, each of us has a pair of holes in the visual fields where the optic nerves penetrate the retina. This blind spot can be demonstrated by passing a small object along the visual horizon until it disappears. However, the visual field is ``seen' by the conscious self as a single unbroken expanse, and this hole is papered over with whatever visual material borders it. If the brain can produce this type of conscious impression in the absence of reality, there is no reason to think that it requires a physiologic reassembly of other stimuli for presentation to a central homunculus. Rather, consciousness may be conceived as a property of the integrated activity of the two cerebral hemispheres and not in need of a separate physical manifestation. Despite this view of consciousness as an ``emergent' property of hemispheric information processing, the hemispheres do require a mechanism for arriving at a singularity of thought and action. If each of the independent information streams in the cortical parallel processor could separately command motor responses, human movement would be a hopeless confusion of mixed activities. A good example is seen in patients in whom the corpus callosum has been transected to prevent spread of epileptic seizures. The brain requires a funnel to narrow down the choices from all of the possible modes of action to the single plan of motor behavior that will be pursued. All cortical regions provide input to the striatum (caudate, putamen, nucleus accumbens, and olfactory tubercle). By constricting all motor responses that are not specifically activated by this system, the basal ganglia ensure a smooth and steady, unitary stream of action.

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Syndromes

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M/E the wall of large bowel consists of 4 layers as elsewhere in the alimentary tract-serosa, muscularis, submucosa and mucosa. The blood supply to the right colon is from the superior mesenteric artery which also supplies blood to the small bowel. The remaining portion of large bowel except the lower part of rectum receives blood supply from inferior mesenteric artery. Anal canal, 3-4 cm long tubular structure, begins at the lower end of the rectum, though is not a part of large bowel, but is included here to cover simultaneously lesions pertaining to this region. Autosomal recessive form with mutation in endothelin-B receptor gene in many other cases. Clinically, the condition manifests shortly after birth with constipation, gaseous distension and sometimes with acute intestinal obstruction. In addition to congenital megacolon discussed above, megacolon may occur from certain acquired causes as under: i. In view of the considerable overlapping of enteritis and colitis, these lesions have already been described under small intestine. Diverticular disease, as it is commonly known, is rare under 30 years of age and is seen more commonly as the age advances. Multiple diverticula of the colon are very common in the Western societies, probably due to ingestion of low-fibre diet but is seen much less frequently in tropical countries and in Japan. Based on the etiologic role of low fibre diet, pathogenesis of diverticular disease of the colon can be explained as under: 1. Increased intraluminal pressure such as due to low fibre content of the diet causing hyperactive peristalsis and thereby sequestration, of mucosa and submucosa. Muscular weakness of the colonic wall at the junction of the muscularis with submucosa. They appear as small, spherical or flaskshaped outpouchings, usually less than 1 cm in diameter, commonly extend into appendices epiploicae and may contain inspissated faeces. M/E the flask-shaped structures extend from the intestinal lumen through the muscle layer. The colonic wall in the affected area is thin and is composed of atrophic mucosa, compressed submucosa and thin or deficient muscularis. The complications of diverticulosis and diverticulitis are perforation, haemorrhage, intestinal obstruction and fistula formation. They are called internal piles if dilatation is of superior haemorrhoidal plexus covered over by mucous membrane, and external piles if they involve inferior haemorrhoidal plexus covered over by the skin. The condition is said to occur in individuals who are habitual users of cathartics of anthracene type. The pathogenesis is obscure but is possibly due to mechanical obstruction of the veins. Anal fissure It is an ulcer in the anal canal below the level of the pectinate line, mostly in midline and posteriorly. Solitary rectal ulcer syndrome It is a condition characterised usually by solitary, at times multiple, rectal ulcers with prolapse of rectal mucosa and development of proctitis. Besides ulceration and inflammation of the rectal mucosa, lamina propria is occupied by spindle-shaped fibroblasts and smooth muscle cells. A classification of polyps, along with benign tumours and malignant tumours, is presented below. Hamartomatous polyps (i) Peutz-Jeghers polyps and polyposis (ii) Juvenile (Retention) polyps and polyposis B. Tubulovillous adenoma (Papillary adenoma, villoglandular adenoma) Familial polyposis syndromes 1. Polyps are much more common in the large intestine than in the small intestine and are more common in the rectosigmoid colon than the proximal colon. G/A Hyperplastic polyps are generally multiple, sessile, smooth-surfaced and small (less than 0. M/E They are composed of long and cystically dilated glands and crypts lined by normal epithelial cells. Hyperplastic polyps are usually symptomless and have no malignant potential unless there is a coexistent adenoma.